Rare Rheumatology News

Disease Profile

Lichen planopilaris

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

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ICD-10

L66.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Follicular lichen planus; Frontal fibrosing alopecia (subtype); Kossard disease;

Categories

Skin Diseases

Summary

Lichen planopilaris (LPP) affects the scalp and hair. It is a form of lichen planus, an inflammatory condition affecting the skin and mucous membranes. Symptoms may include scaly skin and redness around hair follicles, bald patches, and pain, burning, or itching on the scalp. Tiny, red bumps (papules) may appear around hair clusters. LLP can cause scarring which leads to permanent hair loss (cicatricial alopecia). There are 3 forms of LPP which differ by the pattern and location of symptoms: classic LPP, frontal fibrosing alopecia, and Lassueur Graham-Little Piccardi syndrome. The cause of LPP is unknown. It is thought to be an auto-immune disorder of the hair follicles. A diagnosis of LPP is made based on a clinical exam and microscopic examination of a piece of tissue from the affected area. Treatment options may include different oral and topical medications and light therapy.[1][2][3]

Symptoms

The following list includes the most common signs and symptoms in people with lichen planopilaris (LPP). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Symptoms may include:[2][3]

  • Patches of hair loss
  • Scalp redness
  • Scalp pain or burning
  • Scalp itching

Lichen planopilaris usually occurs in adulthood between the ages of 30-60. It affects females more than males. It may occur gradually or suddenly, and it gets worse over time. LPP results in scarred patches on the scalp and permanent hair loss.[1]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Alopecia
Hair loss
0001596
Hyperkeratosis
0000962
Lichenification
0100725
Papule
0200034
30%-79% of people have these symptoms
Abnormal fingernail morphology
Abnormal fingernails
Abnormality of the fingernails

[ more ]

0001231
Dermal atrophy
Skin degeneration
0004334
Pruritus
Itching
Itchy skin
Skin itching

[ more ]

0000989
Skin ulcer
Open skin sore
0200042
5%-29% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters

[ more ]

0008066
Abnormal intestine morphology
Abnormality of the intestine
0002242
Hepatitis
Liver inflammation
0012115
Hypopigmented skin patches
Patchy loss of skin color
0001053
Neoplasm of the oral cavity
0100649
Onycholysis
Detachment of nail
0001806
Pterygium
0001059

Cause

The cause of lichen planopilaris is unknown. It is thought to be an autoimmune disorder in which white blood cells attack and destroy skin and hair cells.[1][3]

Diagnosis

The diagnosis of lichen planopilaris (LPP) is made through a clinical exam and a microscopic examination of a piece of the affected skin.[2] A punch biopsy is usually done to get the piece of skin. LPP may be difficult to diagnose because its symptoms look like other more common conditions.[3]

Treatment

Treatment for lichen planopilaris (LPP) focuses on managing the symptoms and stopping the condition from spreading and getting worse. Different medications are used, including corticosteroids, anti-malarial drugs, antibiotics, and retinoids.[2][4] These can be taken by mouth, injected, or put on the skin. Light therapy is another treatment option. People with LPP often have to try several different treatment methods before the symptoms improve.[5]

One specialist who may be involved in the care of someone with lichen planopilaris is a dermatologist.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

      Organizations Providing General Support

        Learn more

        These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

        Where to Start

        • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
        • The American Osteopathic College of Dermatology (AOCD) has developed an information page on this condition. Click on the link to read more.

          In-Depth Information

          • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
          • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
          • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
          • PubMed is a searchable database of medical literature and lists journal articles that discuss Lichen planopilaris. Click on the link to view a sample search on this topic.

            References

            1. Lepe K, Salazar FJ. Lichen Planopilaris. In: StatPearls. Jan 2020; https://pubmed.ncbi.nlm.nih.gov/29262050.
            2. Babahosseini H, Tavakolpour S, Mahmoudi H, et al. Lichen planopilaris: retrospective study on the characteristics and treatment of 291 patients. J Dermatolog Treat.. 2019; 30(6):598-604. https://pubmed.ncbi.nlm.nih.gov/30411987.
            3. Kerkemeyer KL, Green J. Lichen planopilaris: A retrospective study of 32 cases in an Australian tertiary referral hair clinic. Australas J Dermatol. 2018; 59(4):297-301. https://pubmed.ncbi.nlm.nih.gov/29297588.
            4. Errichetti E, Figini M, Croatto M, Stinco G. Therapeutic management of classic lichen planopilaris: a systematic review. Clin Cosmet Investig Dermatol. 2018; 11:91-102. https://pubmed.ncbi.nlm.nih.gov/29520159.
            5. Randolph MJ, Salhi WA, Tosti A. Lichen Planopilaris and Low-Level Light Therapy: Four Case Reports and Review of the Literature About Low-Level Light Therapy and Lichenoid Dermatosis. Dermatol Ther (Heidelb). 2020; 10(2):311-319. https://pubmed.ncbi.nlm.nih.gov/32060796.
            6. Meinhard J, Stroux A, Lünnemann L, Vogt A, Blume-Peytavi U. Lichen planopilaris: Epidemiology and prevalence of subtypes a retrospective analysis in 104 patients. J Dtsch Dermatol Ges. 2014 Mar; 12(3):229-35, 229-36. https://www.ncbi.nlm.nih.gov/pubmed/24533855.

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